title: [IgE mediated anaphylaxis in a patient with systemic mastocytosis].
abstract: BACKGROUND
  Anaphylaxis is a severe, generalized, life-threatening reaction of rapid onset. We report the case of a patient presenting several systemic anaphylactic reactions over many years, initially ascribed to a cereals allergy but which finally proved to be due to systemic mastocytosis hidden for a long time.
  
  
  METHODS
  A 53-year-old man consulted for an eruption consisting of monomorphic pigmented maculopapular lesions on the trunk associated with itching and urticaria. He was a farmer and presented severe sensitivity to cereals, with anaphylaxis, which continued despite withdrawal of these allergens. Skin and bone marrow infiltration, abnormal mast cells, positivity for c-kit 816 mutation and the persistent elevation of serum tryptase enabled a diagnosis of indolent systemic mastocytosis to be made.
  
  
  CONCLUSIONS
  In systemic mastocytosis anaphylaxis is an expected complication relating to the proliferation of mast cells and a massive increase in mediator release (non-immunological mechanism). All patients with severe and recurrent anaphylaxis should be analyzed for underlying mastocytosis by careful physical examination and assay of baseline tryptase.
authors.affiliation: Service de dermatologie, centre hospitalier de Montauban, 100, rue Léon-Cladel, BP 765, 82013 Montauban cedex, France; Service de dermatologie, hôpital Larrey, université Paul-Sabatier, CHU, 24, chemin de Pouvourville, TSA 30030, 31059 Toulouse cedex 9, France.
authors.forename: H
authors.initials: H
authors.lastname: Escande
authors.forename: I
authors.initials: I
authors.lastname: Bennani
authors.forename: C
authors.initials: C
authors.lastname: Bulai Livideanu
authors.forename: C
authors.initials: C
authors.lastname: Uthurriague
authors.forename: C
authors.initials: C
authors.lastname: Paul
authors.forename: J
authors.initials: J
authors.lastname: Nougué
mesh_terms: D000382:Agricultural Workers' Diseases / Q000209:etiology; D000485:Allergens; D000707:Anaphylaxis / Q000209:etiology / Q000276:immunology*; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D057210:Delayed Diagnosis; D003937:Diagnosis, Differential; D002523:Edible Grain / Q000276:immunology; D006801:Humans; D006969:Hypersensitivity, Immediate / Q000150:complications; D007073:Immunoglobulin E / Q000276:immunology*; D007372:Interferons / Q000627:therapeutic use; D008297:Male; D008407:Mast Cells / Q000473:pathology; D034721:Mastocytosis, Systemic / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000276:immunology*; D008875:Middle Aged; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D012867:Skin / Q000473:pathology; D012882:Skin Tests; D013315:Stress, Psychological / Q000150:complications; D053802:Tryptases / Q000097:blood
chemical_list: D000485:Allergens; D007073:Immunoglobulin E; D007372:Interferons; D019009:Proto-Oncogene Proteins c-kit; D053802:Tryptases
keywords: Anaphylaxie; Anaphylaxis; Mastocytose; Mastocytosis; Tryptase